成人肾小管间质性肾炎和葡萄膜炎综合征的临床病理特征、治疗及预后：一项符合STROBE标准的全国性回顾性研究
Clinicopathologic characteristics, treatment, and outcomes of tubulointerstitial nephritis and uveitis syndrome in adults: A national retrospective strobe-compliant study.
作者信息
Legendre Mathieu, Devilliers Hervé, Perard Laurent, Groh Matthieu, Nefti Habdelamid, Dussol Bertrand, Trad Salim, Touré Fatouma, Abad Sébastien, Boffa Jean-Jacques, Frimat Luc, Torner Stéphane, Seidowsky Alexandre, Massy Ziad André, Saadoun David, Rieu Virginie, Schoindre Yoland, Heron Emmanuel, Frouget Thierry, Lionet Arnaud, Glowacki François, Arnaud Laurent, Mousson Christiane, Besancenot Jean-François, Rebibou Jean-Michel, Bielefeld Philip
机构信息
aNephrology Department, Bocage University Hospital and UMR 1098 bInternal Medicine and Systemic Diseases Department, Bocage University Hospital, Dijon cInternal Medicine Department, Edouard Herriot University Hospital and University Claude Bernard Lyon 1, Lyon dInternal Medicine Department, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin University Hospital and University Paris Descartes, Paris eNephrology Department, Macon Hospital, Mâcon fNephrology Department, La Conception University Hospital and University Aix-Marseille, Marseille gInternal Medicine Department, Ambroise-Paré University Hospital, Paris hNephrology Department, Maison Blanche University Hospital, Reims iInternal Medicine Department, University Hospital Avicenne and University Sorbonne, Paris jNephrology Department, Tenon University Hospital, Paris kNephrology Department, Brabois University Hospital and INSERM CIC-EC CIE6, Nancy lNephrology Department, Dole Hospital, Dôle mNephrology Department, Ambroise-Paré University Hospital, Boulogne-Billancourt nInternal Medicine and Clinical Immunology Department, Pitié Salpetriere University Hospital and DHU Inflammation, Immunopathology, Biotherapy, Paris VI oInternal Medicine Department, University Hospital Clermont Ferrand, Clermont-Ferrand pInternal Medicine Department, Quinze-Vingts Ophthalmogy Hospital, Paris qNephrology Department, University Hospital, Rennes rNephrology Department, University Hospital Claude Huriez, Lille sRheumatology Department, National Referral Center for Rare Autoimmune Diseases, Strasbourg University Hospital, and UMR 1109, Strasbourg, France.
出版信息
Medicine (Baltimore). 2016 Jun;95(26):e3964. doi: 10.1097/MD.0000000000003964.
Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare disease, defined by the association of idiopathic acute TINU. The aim of our work was to determine the characteristics of adult TINU syndrome in France, and to assess factors (including treatment) influencing medium-term prognosis.We conducted a nationwide study including 20 French hospitals. Clinical, laboratory, and renal histopathologic data of 41 biopsy-proven TINU syndromes were retrospectively collected. The patients were diagnosed between January 1, 1999 and December 1, 2015.Twenty-five females and 16 males were included (F/M ratio: 1.6:1). The median age at disease onset was 46.8 years (range 16.8-77.4) with a median serum creatinine level at 207 μmol/L (range 100-1687) and a median estimated glomerular filtration rate (eGFR) at 27 mL/min per 1.73 m (range 2-73). Twenty-nine patients (71%) had a bilateral anterior uveitis and 24 (59%) had deterioration in general health at presentation. Moderate proteinuria was found in 32 patients (78%) (median proteinuria 0.52 g/24 h; range 0.10-2.10), aseptic leukocyturia in 25/36 patients (70%). The evaluation of renal biopsies revealed 41 patients (100%) with an acute tubulointerstitial nephritis, 19/39 patients (49%) with light to moderate fibrosis and 5 patients (12%) with an acute tubular necrosis. Thirty-six patients (88%) were treated with oral corticosteroids. After 1 year of follow-up, the median eGFR was 76 mL/min per 1.73 m (range 17-119) and 32% of the patients suffered from moderate to severe chronic kidney disease. Serum creatinine (P < 0.001, r = -0.54), serum bicarbonate and phosphate levels (respectively, P = 0.01, r = 0.53; and P = 0.04, r = 0.46), and age (P = 0.03, r = -0.37) at the 1st symptoms were associated with eGFR after 1 year. During the 1st year 40% of patients had uveitis relapses. The use of oral corticosteroids was not associated with a better kidney function but was associated with fewer uveitis relapses (P = 0.44 and 0.02, respectively).In our study, 32% of patients were suffering from moderate to severe chronic kidney disease after 1 year of follow-up, and 40% had uveitis relapses during this follow-up. This work also suggests that oral corticosteroids are effective for the treatment of TINU syndrome's uveitis.
肾小管间质性肾炎和葡萄膜炎（TINU）综合征是一种罕见疾病，定义为特发性急性肾小管间质性肾炎和葡萄膜炎的联合病症。我们研究的目的是确定法国成人TINU综合征的特征，并评估影响中期预后的因素（包括治疗）。我们开展了一项全国性研究，纳入了20家法国医院。对41例经活检证实的TINU综合征患者的临床、实验室及肾脏组织病理学数据进行了回顾性收集。这些患者的诊断时间为1999年1月1日至2015年12月1日。纳入25名女性和16名男性（女性/男性比例为1.6:1）。发病时的中位年龄为46.8岁（范围16.8 - 77.4岁），血清肌酐水平中位数为207μmol/L（范围100 - 1687），估算肾小球滤过率（eGFR）中位数为每分钟27 mL/1.73m²（范围2 - 73）。29例患者（71%）患有双侧前部葡萄膜炎，24例患者（59%）在就诊时全身健康状况恶化。32例患者（78%）出现中度蛋白尿（蛋白尿中位数为0.52 g/24小时；范围0.10 - 2.10），36例患者中有25例（70%）出现无菌性白细胞尿。肾脏活检评估显示41例患者（100%）患有急性肾小管间质性肾炎，39例患者中有19例（49%）出现轻度至中度纤维化，5例患者（12%）出现急性肾小管坏死。36例患者（88%）接受了口服糖皮质激素治疗。随访1年后，eGFR中位数为每分钟76 mL/1.73m²（范围17 - 119），32%的患者患有中度至重度慢性肾脏病。首次出现症状时的血清肌酐水平（P < 0.001，r = -0.54）、血清碳酸氢盐和磷酸盐水平（分别为P = 0.01，r = 0.53；P = 0.04，r = 0.46）以及年龄（P = 0.03，r = -0.37）与1年后的eGFR相关。在第1年，40% 的患者出现葡萄膜炎复发。口服糖皮质激素的使用与更好的肾功能无关，但与较少的葡萄膜炎复发相关（分别为P = 0.44和0.02）。在我们的研究中，随访1年后32%的患者患有中度至重度慢性肾脏病，在此随访期间40%的患者出现葡萄膜炎复发。这项研究还表明口服糖皮质激素对TINU综合征的葡萄膜炎治疗有效。
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